Supplementary Material for: Working Under Pressure: Scleroderma Presenting with Bilateral Exudative Retinal Detachment in the Context of Scleroderma Renal Crisis – A Case Report

This is an observational case report to detail a novel case of scleroderma renal crisis presenting as bilateral exudative retinal detachments in a patient with newly diagnosed systemic sclerosis. An otherwise healthy 58-year-old female presented primarily with vision complaints and was found to have malignant hypertension (230/120 mmHg) and bilateral exudative retinal detachment on dilated fundus examination and macular OCT scan. Further history revealed sclerodactyly, mild dysphagia, and dyspnea. She was diagnosed with diffuse Systemic Sclerosis and Sjogren's Syndrome complicated by an episode of scleroderma renal crisis based on initial medical workup. She was admitted to intensive care for management of refractory hypertension with IV antihypertensive therapy. Three-months after treatment, her visual symptoms and ocular findings resolved. The presence of exudative retinal detachment among other signs of hypertensive retinopathy warrants thorough systemic screening for underlying causes of malignant hypertension, including systemic sclerosis. Treatment of the underlying disease with urgent antihypertensive therapy resolved the exudative retinal detachments and restored vision in the case of a scleroderma renal crisis.

本研究为一项观察性病例报告，旨在详细阐述1例新确诊系统性硬化症（Systemic Sclerosis）患者并发硬皮病肾危象（scleroderma renal crisis）、以双侧渗出性视网膜脱离（bilateral exudative retinal detachments）为首发表现的罕见病例。患者为58岁既往体健女性，因视力不适就诊；散瞳眼底检查（dilated fundus examination）及黄斑光学相干断层扫描（macular OCT scan）结果显示，其罹患恶性高血压（血压达230/120 mmHg）与双侧渗出性视网膜脱离。进一步病史问询提示患者存在硬指症（sclerodactyly）、轻度吞咽困难（dysphagia）及呼吸困难（dyspnea）。经初始临床评估，患者被诊断为弥漫性系统性硬化症（diffuse Systemic Sclerosis）合并干燥综合征（Sjogren's Syndrome），且并发硬皮病肾危象。患者随后被收入重症监护病房，接受静脉降压治疗（IV antihypertensive therapy）以控制难治性高血压（refractory hypertension）。治疗3个月后，患者的视力症状及眼部体征完全缓解。高血压性视网膜病变（hypertensive retinopathy）等体征伴随渗出性视网膜脱离的临床表型，提示需对恶性高血压的潜在病因开展全面系统筛查，其中亦涵盖系统性硬化症。本病例证实，针对基础疾病采取紧急降压治疗，可有效缓解硬皮病肾危象患者的渗出性视网膜脱离，恢复患者视力。